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Step III

Step III - Heme 1

QuestionAnswer
What is the typical presentation of anemia Fatigue, loss of energy, light-headedness, SOB, confusion
What other dz can present similar to anemia CO poisoning, methemoglobinemia, hypoxia, ischemic heart dz
What may you find on exam in anemic patient Pallor, jaundice, scleral icterus, pale conjunctiva, low grade systolic flow murmur
What is the most accurate test for anemia Bone marrow biopsy
Which type of anemia is a/w inc RDW Iron defx
What stain is specific for iron Prussian blue
What is happening to iron in sideroblastic anemia Iron build up within mitochondria of RBC
Pt >50 yo with microcytic anemia and (-) FOBT. What is high on suspicion and what do you do next Colon cancer; colonoscopy
Iron studies in pt w/ anemia of chronic dz would show nL/incr iron stores (ferritin/TIBC) b/c of inability to process iron into cells/Hb
Where does extravascular hemolysis occur Liver and spleen (can’t see on peripheral smear)
How does VB12 defx differ from folate defx Neurological problems; MC peripheral neuropathy
What is the best initial test for suspicion of VB12 defx CBC + peripheral smear
What does a peripheral smear show in VB12 defx Hypersegmented neutrophils + oval cells
What other testing should you consider ordering for evaluation of VB12 deficiency LDH + bilirubin level (elevated)
What happens to the reticulocyte count in macrocytic anemia Decreased
What is the most accurate test for VB12 and folate acid defx VB12 level, folate level (low)
Sometimes a pt has nL VB12 level but is actually VB12 defx. Why Transcobalamin is acute phase reactant so when pt in state of stress VB12 level can rise making it seem “nL”
If VB12 levels are nL but you still have high suspicion of VB12 defx. What can you order next Methylmalonic acid level (incr)
What happens to homocysteine levels in VB12 and folate defx Goes UP in BOTH dz; recall that homocysteine becomes methionine via methyl transfer from THF-CH3 to become THF with VB12 as cofactor. Therefore w/o VB12, THF-CH3 and homocysteine builds up
Pt comes in with fatigue and peripheral neuropathy. VB12 level is LOW and MMA is high so you dx pt as VB12 defx. What is the next best test to confirm etio of VB12 defx Anti-parietal + anti-IF Abs to dx pernicious anemia
What is the tx for macrocytic anemia Replacement
What is the Schilling test and what is it also called Older test for finding etio of VB12 defx that involves I-IV stages; VB12 absorption test
What happens in Stages I and II of the Schilling test Stage I: Pt takes PO radioactive VB12 followed by IM VB12 injection 1 hour later; urine VB12 measured over 24hrs Stage II: pt takes IF + radioactive VB12
If Schilling test Stage I is abnL and Stage II is nL. What is your conclusion Pt is VB12 def d/t to stomach unable to make IF
If Schilling test Stage II abnL then you deduce Pt has some dz where IF is not absorbed in small intestine eg Crohns, Celiac, biliary dz, liver/pancreas dz
What happens in Schilling test Stage III and what etio are we looking for Pt takes antibx for weeks then given radioactive VB12; if microflora is preventing IF:VB12 absorption
What happens in Schilling test Stage IV and what etio are we looking for Pt given pancreatic enzymes then radioactive VB12; pancreatic dz
Hemolytic anemia is characterized by what pt presention SUDDEN ONSET fatigue + weakness +/- jaundice, dark urine
What screening labs are ordered (and findings) for work up of hemolytic anemia Peripheral smear, Incr indirect bili, incr LDH, incr retic count, decr haptoglobin
What is the function(s) of haptoglobin and decreased haptoglobin comes from what etio Hb binding protein, acute phase reactant; hemolytic anemia (blood stream consuming haptoglobin), liver dz (not making haptoglobin), drugs (OCP, quinidine, INH)
Normal haptoglobin levels indicate what etios Extravascular hemolysis or non hemolytic anemia
nL haptoglobin + incr retic = extravascular hemolysis @ liver/spleen
nL haptoglobin + not high retic = non hemolytic cause of anemia
low haptoglobin w/o signs of hemolytic anemia = liver not making haptoglobin
What conditions may elevate haptoglobin Inflammatory states (UC, MI, infection)
What is hemosiderin Oxidized product of Hb
Intravascular hemolysis diagnostic tests will show Hb in urine, hemosiderin in urine, fragmented cells/schistocytes/helmet cells on peri smear
What type of anemia presents as very severe chest, thigh and back pain Sickle cell anemia (type of hemolytic anemia)
What are some possible physical exam findings for pts w/ sickle cell anemia Retinal infarction, stroke/prior stroke, flow murmur, lung consolidation, splenomegaly in kids/absent spleen in adults, skin ulcers, aseptic necrosis of hip
Pt w/ severe chest, thigh, and back pain. Suspect he has sickle cell. Next best step O2, IV nL saline, pain meds
Pt w/ severe chest, thigh, back pain and FEVER. Suspect he has sickle cell. In addition to O2, fluids, analgesia, what else would you give and why Ceftriaxone, levaquin, moxifloxacin; sickle cell pts lack a spleen and in infection states this measure is URGENT
Sickle cell pt comes in with fever. What do you give first and what screening tests do you order Antibx; CBC, UA, blood cx, CXR, retic count
When is an exchange transfusion the answer to tx for sickle cell patient Visual changes from retinal infarct, pleuritic pain from pulm infarct, priapism from prostatic plexus infarct, stroke
Pt w/sickle cell has sudden drop in hematocrit while in hospital over 2 days. Dx, most accurate test, tx Parvovirus B19 or folate defx; PCR DNA of virus; folate replacement (if not already) + transfuse w/ IV Igs
Where does parvovirus B19 invade in sickle cell pts Bone marrow hence cessation of cell production
Pt comes into hospital with sickle cell crisis. Past hx shows crises at rate of 4+/year. You tx and now plan for D/C. What do you prescribe Folate replacement, pneumococcal vaccx, hydroxyurea (for prevention of future attacks)
Pt c/o visual disturbances and dysuria. Denies pain and states this has happened x1 this year before. UA shows UTI w/ RBCs. Dx Hb sickle cell disease (milder than SC, lacks painful crises)
Sickle trait pts usually present in what way Renal issues = hematuria and difficulty concentrating urine
Pts with autoimmune hemolysis usually have what kind of medical history Other autoimmune dz (CLL, lymphoma, rh arthrx, SLE) or drugs (PCN, quinine, sulfa, alpha methydopa)
What are findings of diagnostic testing in pts with autoimmune hemolysis Same as for hemolytic anemia in general: incr retic count/LDH/indirect bili; exception is that intravascular and extravascular forms of this dz will show DECR haptoglobin
What does the peri smear show in pts w/ autoimm hemolysis Spherocytes
What is the most accurate test for autoimm hemolysis and what does it show Coomb’s test; warm Abs (IgG only)
What is best initial tx for autoimm hemolysis Steroids
What is the most effective tx for recurrent episodes of autoimm hemolysis Splenectomy
What can you do next if pt w/ autoimm hemolysis does not respond to steroids and blood transfusions IV Ig
Which type of Abs are the only Abs that respond to steroids and splenectomy in pt with autoimm hemolysis Warm IgG
Created by: DrINFJ